J Clin Immunol. 2022 Jun 14. doi: 10.1007/s10875-022-01297-3. Online ahead of print.

Maria Christine Ernst Andersen ¹, Stine Dydensborg Sander ¹, Gunvor Madsen ², Søren T Lillevang ³, Joseph Murray ⁴, Steffen Husby ⁵

Author information

¹Hans Christian Andersen Children's Hospital, Odense University Hospital, Kloevervaenget 23C, 5000, Odense C, Denmark.

²Department of Pathology, Odense University Hospital, Odense, Denmark.

³Department of Clinical Immunology, Odense University Hospital, Odense, Denmark.

⁴Division of Gastroenterology, Mayo Clinic, Rochester, MN, USA.

⁵Hans Christian Andersen Children's Hospital, Odense University Hospital, Kloevervaenget 23C, 5000, Odense C, Denmark. steffen.husby@rsyd.dk.

Abstract

Purpose: Selective IgA deficiency (IgAD) is the most common primary immunodeficiency, frequently leading to only minor clinical complaints. IgAD may be associated with autoimmune diseases such as celiac disease (CeD). Although IgAD is thought to precede CeD and autoimmunity, the association between the two conditions has not been clarified.

Methods: Routine techniques were used to measure serum IgA and celiac diagnostic markers as transglutaminase 2 IgA (TG2-IgA) and deamidated gliadin IgG and for immunohistochemistry for IgG, IgM, and IgA.

Results: We report two childhood cases of complete IgA deficiency that evolved after the diagnosis of CeD and the start of a gluten-free diet. Histology showed persistence of IgA in the intestinal mucosa.

Conclusion: Both children with CeD showed IgA deficiency that unexpectedly developed after the initiation of a gluten-free diet. This supports IgA deficiency as a process that develops gradually and occurs due to specific defects in immunoregulation.